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Disease Profile

Hemorrhagic shock and encephalopathy syndrome

Prevalence estimates on Rare Medical Network websites are calculated based on data available from numerous sources, including US and European government statistics, the NIH, Orphanet, and published epidemiologic studies. Rare disease population data is recognized to be highly variable, and based on a wide variety of source data and methodologies, so the prevalence data on this site should be assumed to be estimated and cannot be considered to be absolutely correct.


US Estimated

Europe Estimated

Age of onset





Autosomal dominant A pathogenic variant in only one gene copy in each cell is sufficient to cause an autosomal dominant disease.


Autosomal recessive Pathogenic variants in both copies of each gene of the chromosome are needed to cause an autosomal recessive disease and observe the mutant phenotype.


dominant X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.


recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder.


Mitochondrial or multigenic Mitochondrial genetic disorders can be caused by changes (mutations) in either the mitochondrial DNA or nuclear DNA that lead to dysfunction of the mitochondria and inadequate production of energy.


Multigenic or multifactor Inheritance involving many factors, of which at least one is genetic but none is of overwhelming importance, as in the causation of a disease by multiple genetic and environmental factors.


Not applicable


Other names (AKA)

HSES; Hemorrhagic shock and encephalopathy syndrome


Blood Diseases


Hemorrhagic shock and encephalopathy syndrome (HSES) is a rare disease that occurs suddenly in previously healthy children. This condition is characterized by severe shock, impairment of the blood's ability to clot (coagulopathy), abnormal brain function or structure (encephalopathy), and liver and kidney dysfunction. Most cases of HSES occur in infants from age 3 to 8 months of age, although it can also occur in older children. Individuals with HSES have extremely high body temperatures and multiple organ failures. This condition often causes long term neurological problems or death. The cause of the HSES is unknown. There is no cure for HSES; however, different methods can be used to try to manage associated symptoms.[1][2]


The cause of HSES is unknown.[3] Some researchers believe that this condition is caused by a complex combination of genetic and environmental factors.[4][2] Researchers have proposed various factors that may contribute to the development of this condition, including infection, exposure to toxins in the environment, and overwrapping of infants with a fever.[1]

Learn more

These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

In-Depth Information

  • The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
  • PubMed is a searchable database of medical literature and lists journal articles that discuss Hemorrhagic shock and encephalopathy syndrome. Click on the link to view a sample search on this topic.


  1. Palumbo EJ. Hemorrhagic shock and encephalopathy syndrome. Merck Manual for Healthcare Professionals. May 2014; https://www.merckmanuals.com/professional/pediatrics/miscellaneous-disorders-in-infants-and-children/hemorrhagic-shock-and-encephalopathy-syndrome-hses.
  2. Ichiro Kuki, Masashi Shiomi, Shin Okazaki, Hisashi Kawawaki, Kiyotaka Tomiwa, Kiyoko Amo, Masao Togawa, Junichi Ishikawa, Hiroshi Rinka. Characteristic Neuroradiologic Features in Hemorrhagic Shock and Encephalopathy Syndrome. Journal of Child Neurology. Mar 2015; 30(4):468-475. https://www.ncbi.nlm.nih.gov/pubmed/?term=25512363.
  3. Rinka H, Yoshida T, Kubota T, Tsuruwa M, Fuke A, Yoshimoto A, Kan M, Miyazaki D, Arimoto H, Miyaichi T, Kaji A, Miyamoto S, Kuki I, Shiomi M. Hemorrhagic shock and encephalopathy syndrome--the markers for an early HSES diagnosis. BMC Pediatr. October 16, 2008; 8:43. https://www.ncbi.nlm.nih.gov/pubmed/18922188. Accessed 5/19/2009.
  4. S Sofer, B Yerushalmi, E Shahak, T Berenstein, and H Schulman. Possible aetiology of haemorrhagic shock and encephalopathy syndrome in the Negev area of Israel. Archives of Disease in Childhood. October 1996; 75:4. https://www.pubmedcentral.nih.gov/articlerender.fcgi?artid=1511758. Accessed 5/19/2009.

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